Respiratory syncytial virus infections in pediatric transplant recipients: A Canadian Paediatric Surveillance Program study.

The incidence and spectrum of severity of RSV infections in SOT or HSCT recipients is not known. From September 2010 through August 2013, pediatricians were surveyed monthly by the CPSP for SOT or HSCT recipients with RSV infection within two yr post-transplant. There were 24 completed case report forms that fit the inclusion criteria (10 HSCT and 14 SOT recipients). Six of 24 cases (25%) remained outpatients, and 11 (46%) were managed on an inpatient ward, while seven (29%) required intensive care of which five required mechanical ventilation and two died of RSV infection. Ten of 23 cases (43%) were nosocomial with these data not recorded for one case. Many transplant recipients recover uneventfully from RSV infection in the first two yr post-transplant. However, severe disease and death also occur. Larger studies are required to establish risk factors for poor outcomes. Prevention of nosocomial RSV should be a priority in transplant recipients.

Performance of acute flaccid paralysis surveillance compared with World Health Organization standards.

AIM: To compare acute flaccid paralysis (AFP) surveillance systems used by members of the International Network of Paediatric Surveillance Units (INoPSU) across the five AFP surveillance performance indicators recommended by the World Health Organization (WHO) for the maintenance of polio-free certification. METHODS: A survey was administered to AFP surveillance co-ordinators in five INoPSU member countries (Australia, Belgium, Canada, New Zealand and Switzerland). Data collected included information on surveillance system processes, WHO-recommended performance indicators, investigative practices and final diagnoses of cases from 2006 to 2010. RESULTS: All countries contacted completed the survey. Each country used similar case definitions and processes for collecting AFP data. All countries used at least one of the WHO indicators for surveillance. No country consistently met the performance indicator for incidence or stool sampling. In all countries, at least one form of neurological testing was used to diagnose cases of AFP. Guillain-Barré syndrome was the most common final diagnosis in all countries for all years examined. CONCLUSIONS: Industrialised countries surveyed do not consistently meet the WHO-recommended AFP surveillance performance indicators. An opportunity exists for INoPSU to suggest a standard way for member countries to collect AFP data in order to examine the potential for strengthening the current systems or introducing additional enterovirus surveillance or alternative/complementary neurological performance measures suitable for countries that have eliminated polio. INoPSU member countries are evaluating these possibilities.

Severe combined immunodeficiency (SCID) in Canadian children: a national surveillance study.

PURPOSE: Severe Combined Immune Deficiency (SCID) is universally fatal unless treated with hematopoietic stem cell transplantation (HSCT). Following the identification of disseminated Bacille Calmette-Guérin (BCG) infections in Canadian First Nations, Métis and Inuit (FNMI) children with unrecognized primary immune deficiencies, a national surveillance study was initiated in order to determine the incidence, diagnosis, treatment and outcome of children with SCID in Canada. METHODS: Canadian pediatricians were asked to complete a monthly reporting form if they had seen a suspected SCID case, from 2004 to 2010, through the Canadian Paediatric Surveillance Program (CPSP). If the case met CPSP SCID criteria, more detailed data, including demographics and clinical information about investigations, treatment and outcome was collected. RESULTS: A total of 40 cases of SCID were confirmed for an estimated incidence of SCID in non-FNMI Canadian children of 1.4 per 100,000 live births (95 % CI 1 to 1.9/100,000). The proportion of SCID cases that were FNMI (17.5 %) was almost three times higher than was expected on the basis of proportion of the pediatric population estimated to be FNMI (6.3 %) resulting in an estimated incidence of 4.4 per 100,000 live births (95 % CI 2.1 to 9.2/100,000) in FNMI Canadian children. The mean age at diagnosis for all SCID cases was 4.2 months (range 1­583 days). There were 12 deaths (30 %; 95 % CI 18­46 %); seven died of confirmed or suspected infections before they could receive an HSCT. CONCLUSIONS: The frequency of SCID cases in FNMI children is higher than in the general Canadian pediatric population. The high mortality rate, due primarily to infection, suggests that early diagnosis by newborn screening followed by HSCT could significantly benefit children with SCID.

Clinical characteristics of pediatric myasthenia: a surveillance study.

OBJECTIVE: To evaluate the incidence, clinical features, diagnostic, and treatment trends of pediatric myasthenia in Canada. METHODS: Through established Canadian Pediatric Surveillance Program methodology, physicians were anonymously surveyed for cases of pediatric myasthenia using a standardized clinical questionnaire containing deidentified data. Inclusion criteria were any child <18 years old with ≥1 of the following: (1) fluctuating ptosis or extraocular weakness, (2) skeletal muscle weakness or fatigue, and (3) any of the following supportive tests: clinical response to acetylcholinesterase inhibitor, positive antibodies, abnormal slow repetitive nerve stimulation, or single-fiber electromyography. RESULTS: In 2 years of surveillance, 57 confirmed cases were reported. There were 34 generalized and 18 ocular reports of juvenile myasthenia gravis plus 5 congenital myasthenic syndrome cases. There were 14 incident cases in 2010 and 6 in 2011. Age of onset ranged from "birth" to 17 years for the generalized form compared with 18 months to 11 years for the ocular subtype. Positive acetylcholine receptor titers were found in 22 (67%) of 33 generalized cases and 8 (44%) of 18 ocular patients. Of patients started on pyridostigmine, improvement was noted in 33 (100%) of 33 generalized cases and 15 (88%) of 17 ocular cases. CONCLUSIONS: This study represents the largest descriptive series of pediatric myasthenia in North America and provides valuable information about clinical characteristics. A high index of suspicion is important for this treatable disease. Children generally respond promptly to readily available therapies.

Canadian allergists' and nonallergists' perception of epinephrine use and vaccination of persons with egg allergy.

BACKGROUND: Studies suggest knowledge gaps about epinephrine use and vaccination of persons with egg allergy. OBJECTIVE: We compared the perception of Canadian allergists and nonallergists on issues related to epinephrine use and vaccination of persons with egg allergy. METHODS: Canadian allergists, pediatricians, general practitioners/family physicians and emergency room physicians were recruited through medical associations and surveyed on these issues. Multivariate logistic regression models were used to identify determinants of specific responses. RESULTS: One-hundred fourteen allergists and 613 nonallergists participated. For most issues with accepted best practices, allergists were more likely to adhere to recommendations. Allergists versus nonallergists were more likely to recommend intramuscular epinephrine for anaphylaxis (odds ratio [OR] = 3.8; 95% CI, 1.43-10.11). Older physicians (OR = 0.98; 95% CI, 0.97-0.99), Canadian-Paediatric-Surveillance-Program participants (OR = 0.48; 95% CI, 0.24-0.96), family physicians (OR = 0.39; 95% CI, 0.16-0.96), and general practitioners (OR = 0.14; 95% CI, 0.04-0.52) were less likely to recommend intramuscular use. However, in severe anaphylaxis, >25% of both groups would not give epinephrine for patients presenting with breathing difficulties or hypotension. Use of epinephrine for severe anaphylaxis was less likely in older physicians (OR = 0.97; 95% CI, 0.95-0.99), female physicians (OR = 0.60; 95% CI, 0.39-0.89), and those practicing in Ontario (OR = 0.56; 95% CI, 0.36-0.86), Manitoba (OR = 0.42; 95% CI, 0.19-0.90), or Nova-Scotia (OR = 0.31; 95% CI, 0.12-0.78). Allergists (OR = 6.22; 95% CI, 3.60-10.72) and physicians treating mainly children (OR = 3.41; 95% CI, 1.87-6.25), or practicing in Quebec (OR = 1.68; 95% CI, 1.12-2.55) were more likely to recommend measles-mumps-rubella vaccination in a community facility. CONCLUSION: Knowledge gaps about mode and indications for epinephrine administration and vaccination policies need to be addressed in future education programs to ensure prompt epinephrine use and to avoid unnecessary restriction of vaccines.

Transfusion-related acute lung injury in the Canadian paediatric population.

BACKGROUND: The incidence of transfusion-related acute lung injury (TRALI) in adults is approximately one per 5000 transfusions. The Canadian Paediatric Surveillance Program undertook the present study to determine the incidence of TRALI in the paediatric population and to describe the characteristics and outcomes of children with TRALI. METHODS: The present surveillance study was conducted over a three-year period. RESULTS: Four TRALI cases were reported, yielding an incidence rate of 1.8 per 100,000 transfusions. The degree of severity varied: in two patients, only supplemental oxygen was necessary, while the other two required mechanical ventilation. CONCLUSION: TRALI was reported much less often in the present study compared with adult studies; therefore, it needs to be determined whether TRALI occurs less frequently in children, or alternatively, whether TRALI is recognized less often in children. The possibility that neonates who undergo cardiac surgery are at greater risk of TRALI than other patients should be addressed in future studies.

HISTORIQUE: L'incidence de syndrome respiratoire aigu post transfusionnel (TRALI) est d'environ un cas sur 5 000 transfusions chez les adultes. Le Programme canadien de surveillance pédiatrique (PCSP) a entrepris cette étude pour déterminer l'incidence de TRALI dans la population pédiatrique et pour décrire les caractéristiques et le sort des enfants qui ont un TRALI. MÉTHODOLOGIE: Les chercheurs ont mené l'étude de surveillance pendant trois ans. RÉSULTATS: Quatre cas de TRALI ont été signalés, pour une incidence de 1,8 cas sur 100 000 transfusions. Le degré de gravité variait : deux patients n'ont eu besoin que d'oxygène d'appoint, tandis que les deux autres ont eu besoin d'une ventilation mécanique. CONCLUSION: Dans le cadre de cette étude, le TRALI était beaucoup moins signalé que dans les études auprès d'adultes. Il faut donc déterminer si le TRALI est moins fréquent ou s'il est moins dépisté chez les enfants. Lors de futures études, il faudra évaluer la possibilité que les nouveau-nés qui subissent une chirurgie cardiaque soient plus vulnérables au TRALI que les autres patients.

The Canadian Paediatric Surveillance Program: a framework for the timely data collection on head injury secondary to suspected child maltreatment.

The extent and nature of head injury secondary to child maltreatment in Canada is inadequately understood. These cases, although reasonably rare, are of great clinical importance as a large proportion of them result in death or permanent neurologic deficits. Internationally, published incidence data may underestimate the extent of the problem because of the failure of professionals to recognize and report inflicted head injury. National data on head injury secondary to suspected child maltreatment can inform educational efforts for early detection, guide prevention strategies, and be a foundation for the development of policies and programs to effectively address the needs of victims and their families. The Canadian Paediatric Surveillance Program is described as a system for active surveillance of uncommon high-impact conditions and is ideally suited to the collection of data on head injury secondary to child maltreatment.